Intravenous immunoglobulins improve the function and ameliorate joint involvement in systemic sclerosis: a pilot study

Background: In systemic sclerosis ( SSc), joint involvement may reduce the functional capacity of the hands. Intravenous immunoglobulins have previously been shown to benefit patients with SSc. Aim: To verify the efficacy of intravenous immunoglobulins on joint involvement and function in SSc. Patients and methods: 7 women with SSc, 5 with limited and 2 with diffuse SSc, with a severe and refractory joint involvement were enrolled in the study. Methotrexate and cyclophosphamide pulse therapy did not ameliorate joint symptoms. Hence, intravenous immunoglobulins therapy was prescribed at a dosage of 2 g/ kg body weight during 4 days/ month for six consecutive courses. The presence of joint tenderness and swelling, and articular deformities ( due to primary joint involvement and not due to skin and subcutaneous changes) were evaluated. Before and after 6 months of treatment, patients were subjected to ( 1) Ritchie Index ( RI) evaluation of joint involvement; ( 2) Dreiser Algo- Functional Index ( IAFD) evaluation of hand joint function; ( 3) pain visual analogue scale ( VAS) to measure joint pain; ( 4) Health Assessment Questionnaire ( HAQ) to evaluate the limitations in everyday living and physical disability; and ( 5) modified Rodnan Skin Score for skin involvement. Results: After 6 months of intravenous immunoglobulins therapy, joint pain and tenderness, measured with the VAS, decreased significantly ( p < 0.03), and hand function ( IAFD) improved significantly ( p < 0.02), together with the quality of life ( HAQ; p < 0.03). All patients significantly improved, except for one. The skin score after 6 months of intravenous immunoglobulins therapy was significantly reduced ( p < 0.003). Conclusion: This pilot study suggests that intravenous immunoglobulins may reduce joint pain and tenderness, with a significant recovery of joint function in patients with SSc with severe and refractory joint involvement. The cost of intravenous immunoglobulins might limit their use only to patients who failed disease- modifying antirheumatic drugs.